ZES
MCID: ZLL002
MIFTS: 55

Zollinger-Ellison Syndrome (ZES)

Categories: Cancer diseases, Endocrine diseases, Gastrointestinal diseases, Rare diseases

Aliases & Classifications for Zollinger-Ellison Syndrome

MalaCards integrated aliases for Zollinger-Ellison Syndrome:

Name: Zollinger-Ellison Syndrome 12 73 20 58 36 54 44 15 17 70
Gastrinoma 20 58 70
Pancreatic Ulcerogenic Tumor Syndrome 20
Z E Syndrome 20
Zes 20

Characteristics:

Orphanet epidemiological data:

58
zollinger-ellison syndrome
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe),1-9/100000 (Europe),1-9/1000000 (Worldwide); Age of onset: Adult; Age of death: normal life expectancy;

Classifications:

Orphanet: 58  
Rare gastroenterological diseases
Rare endocrine diseases


Summaries for Zollinger-Ellison Syndrome

KEGG : 36 Zollinger-Ellison syndrome (ZES) is a rare endocrinopathy caused by tumors of the pancreas and duodenum. These tumors, called gastrinomas, release gastrin to produce large amounts of acid that result in severe gastroesophageal peptic ulcer disease and diarrhea. Most ZES cases are sporadic, but about over 20 percent are caused by an inherited genetic disorder called multiple endocrine neoplasia type 1 (see H00247). The clinical presentation is not specific for this disease and there is overlap of symptoms similar to those of a peptic ulcer. The most common symptoms include abdominal pain and diarrhea, sometimes accompanied by heartburn, nausea, and weight loss. Peptic ulceration complicated by bleeding is present in 25% of patients, and is more frequently in patients with sporadic ZES than in those with MEN1. In addition, the gastrinomas may be cancerous. The cancer can be spread to other parts of the body, most commonly to regional lymph nodes and the liver. The treatment of the ZES includes surgical removal and medical management of gastric acid hypersecretion for the prevention of malignant transformation and the genesis of complications.

MalaCards based summary : Zollinger-Ellison Syndrome, also known as gastrinoma, is related to multiple endocrine neoplasia and gastrinoma, and has symptoms including dyspepsia An important gene associated with Zollinger-Ellison Syndrome is GAST (Gastrin), and among its related pathways/superpathways are Gastric acid secretion and Signaling by GPCR. The drugs Dexlansoprazole and Lansoprazole have been mentioned in the context of this disorder. Affiliated tissues include pancreas, pituitary and lymph node, and related phenotypes are zollinger-ellison syndrome and peptic ulcer

Disease Ontology : 12 A syndrome that is characterized by the development of a one or more tumors (gastrinoma) in the pancreas, duodenum, or both that secrete excessive levels of gastrin, a hormone that stimulates production of acid by the stomach.

GARD : 20 Zollinger-Ellison syndrome (ZES) is a condition in which tumors called gastrinomas in the pancreas and duodenum (part of the small intestine) cause high levels of the hormone gastrin in the blood. High levels of gastrin then cause production of too much stomach acid. Signs and symptoms may include abdominal pain, peptic ulcers, vomiting blood, and diarrhea. The tumors are sometimes cancerous and may spread to other areas of the body. In most cases, the cause of ZES is unknown. However, about 25-30% of gastrinomas are caused by an inherited condition called multiple endocrine neoplasia type 1 (MEN1). Treatment for ZES may include medication to reduce the production of stomach acid, and surgery for peptic ulcers or to remove tumors.

Wikipedia : 73 Zollinger-Ellison syndrome (Z-E syndrome) is a disease in which tumors cause the stomach to produce too... more...

Related Diseases for Zollinger-Ellison Syndrome

Diseases related to Zollinger-Ellison Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 331)
# Related Disease Score Top Affiliating Genes
1 multiple endocrine neoplasia 32.1 SST SCT MEN1 GAST CHGA
2 gastrinoma 31.9 SST SCT MEN1 GRP GAST CHGA
3 multiple endocrine neoplasia, type i 31.5 SST SCT MEN1 GHRH GAST CHGA
4 esophagitis 31.5 NTS HRH2 GAST
5 hyperparathyroidism 31.4 SCT MEN1 GAST CHGA
6 gastrointestinal ulceration, recurrent, with dysfunctional platelets 31.3 HRH2 GAST EGF
7 diarrhea 31.0 SST SCT NTS MLN GAST
8 duodenal ulcer 30.8 SST SCT MLN HRH2 GRP GAST
9 adenoma 30.8 SST MEN1 GHRH CHGA
10 insulinoma 30.8 SST SCT MEN1 GAST CHGA
11 primary hyperparathyroidism 30.8 MEN1 GHRH GAST CHGA
12 gastric ulcer 30.7 SST HRH2 GAST EGF
13 pituitary tumors 30.6 SST MEN1
14 mixed ductal-endocrine carcinoma 30.6 GAST CHGA
15 pituitary adenoma, prolactin-secreting 30.5 SST MEN1 CHGA
16 acromegaly 30.5 SST MEN1 GHRH
17 hemangioma 30.4 MEN1 GAST CHGA
18 islet cell tumor 30.4 SST SCT MEN1 GHRH GAST CHGA
19 pituitary adenoma 30.4 SST MEN1 GHRH CHGA
20 conn's syndrome 30.4 SST MEN1 CHGA
21 glucagonoma 30.4 SST SCT GAST CHGA
22 carcinoid tumors, intestinal 30.3 SST MEN1 GAST CHGA
23 pancreatic endocrine carcinoma 30.3 SST MEN1 GAST CHGA
24 carcinoid syndrome 30.3 SST NTS MEN1 GHRH CHGA
25 pyloric stenosis 30.2 SST MLN GAST
26 atrophic gastritis 30.2 SST MEN1 GAST EGF CHGA
27 barrett esophagus 30.2 GAST ATP4A ATP12A
28 ectopic cushing syndrome 30.2 SST CHGA
29 gastritis 30.2 SST SCT HRH2 GAST CHGA ATP4A
30 autoimmune atrophic gastritis 30.1 GAST CHGA
31 irritable bowel syndrome 30.1 SST MLN CHGA
32 peptic ulcer disease 30.1 SST SCT MEN1 HRH2 GRP GAST
33 duodenitis 30.1 GAST ATP4A ATP12A
34 peptic esophagitis 30.0 MLN HRH2 GAST ATP4A ATP12A
35 gastrojejunal ulcer 29.9 NTS ATP4A ATP12A
36 gastric neuroendocrine neoplasm 29.9 SST MEN1 GAST CHGA
37 short bowel syndrome 29.9 SST NTS GAST EGF
38 neuroendocrine carcinoma 29.9 SST MEN1 GRP GAST CHGA
39 somatostatinoma 29.8 SST MEN1 GHRH GAST CHGA
40 tropical sprue 29.8 GAST ATP4A ATP12A
41 vitamin b12 deficiency 29.8 GAST ATP4A ATP12A
42 laryngitis 29.8 HRH2 ATP4A ATP12A
43 peptic ulcer perforation 29.8 HRH2 ATP4A ATP12A
44 active peptic ulcer disease 29.7 SST HRH2 ATP4A ATP12A
45 dumping syndrome 29.7 SST SCT NTS MLN GAST
46 lactose intolerance 29.6 ATP4A ATP12A
47 intestinal obstruction 29.6 MLN ATP4A ATP12A
48 pernicious anemia 29.6 SST SCT GAST CHGA ATP4A ATP12A
49 hyperpituitarism 29.6 SST MEN1 GHRH
50 celiac disease 1 29.6 SCT GAST ATP4A ATP12A

Graphical network of the top 20 diseases related to Zollinger-Ellison Syndrome:



Diseases related to Zollinger-Ellison Syndrome

Symptoms & Phenotypes for Zollinger-Ellison Syndrome

Human phenotypes related to Zollinger-Ellison Syndrome:

58 31 (show all 35)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 zollinger-ellison syndrome 58 31 obligate (100%) Obligate (100%) HP:0002044
2 peptic ulcer 58 31 hallmark (90%) Very frequent (99-80%) HP:0004398
3 esophagitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0100633
4 duodenal ulcer 58 31 hallmark (90%) Very frequent (99-80%) HP:0002588
5 episodic abdominal pain 58 31 hallmark (90%) Very frequent (99-80%) HP:0002574
6 nausea 58 31 hallmark (90%) Very frequent (99-80%) HP:0002018
7 weight loss 58 31 frequent (33%) Frequent (79-30%) HP:0001824
8 jaundice 58 31 occasional (7.5%) Occasional (29-5%) HP:0000952
9 multiple lipomas 58 31 occasional (7.5%) Occasional (29-5%) HP:0001012
10 hyperparathyroidism 58 31 occasional (7.5%) Occasional (29-5%) HP:0000843
11 hypercalcemia 58 31 occasional (7.5%) Occasional (29-5%) HP:0003072
12 erythema 58 31 occasional (7.5%) Occasional (29-5%) HP:0010783
13 hematochezia 58 31 occasional (7.5%) Occasional (29-5%) HP:0002573
14 intestinal obstruction 58 31 occasional (7.5%) Occasional (29-5%) HP:0005214
15 lipoma 58 31 occasional (7.5%) Occasional (29-5%) HP:0012032
16 growth hormone excess 58 31 occasional (7.5%) Occasional (29-5%) HP:0000845
17 pituitary growth hormone cell adenoma 58 31 occasional (7.5%) Occasional (29-5%) HP:0011760
18 pituitary prolactin cell adenoma 58 31 occasional (7.5%) Occasional (29-5%) HP:0006767
19 adrenocortical adenoma 58 31 occasional (7.5%) Occasional (29-5%) HP:0008256
20 extrahepatic cholestasis 58 31 occasional (7.5%) Occasional (29-5%) HP:0012334
21 thyroid adenoma 58 31 occasional (7.5%) Occasional (29-5%) HP:0000854
22 elevated circulating parathyroid hormone level 58 31 occasional (7.5%) Occasional (29-5%) HP:0003165
23 pituitary corticotropic cell adenoma 58 31 occasional (7.5%) Occasional (29-5%) HP:0008291
24 increased urinary cortisol level 58 31 occasional (7.5%) Occasional (29-5%) HP:0012030
25 pituitary null cell adenoma 58 31 occasional (7.5%) Occasional (29-5%) HP:0011761
26 parathyroid hyperplasia 58 31 occasional (7.5%) Occasional (29-5%) HP:0008208
27 glucagonoma 58 31 occasional (7.5%) Occasional (29-5%) HP:0030404
28 increased glucagon level 58 31 occasional (7.5%) Occasional (29-5%) HP:0030688
29 increased circulating cortisol level 31 occasional (7.5%) HP:0003118
30 adrenocortical carcinoma 58 31 very rare (1%) Very rare (<4-1%) HP:0006744
31 gastrointestinal hemorrhage 58 Occasional (29-5%)
32 diarrhea 58 Very frequent (99-80%)
33 hypercortisolism 58 Occasional (29-5%)
34 neuroendocrine neoplasm 58 Obligate (100%)
35 pituitary adenoma 58 Occasional (29-5%)

UMLS symptoms related to Zollinger-Ellison Syndrome:


dyspepsia

GenomeRNAi Phenotypes related to Zollinger-Ellison Syndrome according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability GR00249-S 9.4 HRH2 MLN
2 Decreased viability GR00381-A-1 9.4 GRP HRH2 MLN
3 Decreased viability GR00381-A-3 9.4 GRP HRH2 MLN
4 Decreased viability GR00386-A-1 9.4 GRP MLN
5 Decreased viability GR00402-S-2 9.4 GRP MLN

MGI Mouse Phenotypes related to Zollinger-Ellison Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 digestive/alimentary MP:0005381 9.7 ATP12A ATP4A EGF GAST HRH2 MEN1
2 endocrine/exocrine gland MP:0005379 9.5 ATP4A CHGA EGF GAST GHRH HRH2
3 homeostasis/metabolism MP:0005376 9.32 ATP12A ATP4A CHGA GAST GHRH GRP

Drugs & Therapeutics for Zollinger-Ellison Syndrome

Drugs for Zollinger-Ellison Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 97)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dexlansoprazole Approved, Investigational Phase 4 138530-94-6, 103577-45-3 9578005
2
Lansoprazole Approved, Investigational Phase 4 103577-45-3 3883
3 Proton Pump Inhibitors Phase 4
4 Anti-Ulcer Agents Phase 4
5 Antacids Phase 4
6
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
7
Esomeprazole Approved, Investigational Phase 3 161973-10-0, 161796-78-7, 119141-88-7 4594 9568614
8
Fluorouracil Approved Phase 2, Phase 3 51-21-8 3385
9
Levoleucovorin Approved, Investigational Phase 2, Phase 3 68538-85-2 149436
10
leucovorin Approved Phase 2, Phase 3 58-05-9 6006
11
Oxaliplatin Approved, Investigational Phase 2, Phase 3 61825-94-3 5310940 9887054 43805 6857599
12
Bevacizumab Approved, Investigational Phase 2, Phase 3 216974-75-3
13
Folic acid Approved, Nutraceutical, Vet_approved Phase 2, Phase 3 59-30-3 6037
14 Pancreatic Polypeptide Investigational Phase 2, Phase 3 59763-91-6
15 Cola Phase 3
16 Anesthetics Phase 3
17 Narcotics Phase 3
18 Analgesics, Opioid Phase 3
19 Analgesics Phase 3
20 Anesthetics, Intravenous Phase 3
21 Anesthetics, General Phase 3
22 Vitamin B9 Phase 2, Phase 3
23 Nutrients Phase 2, Phase 3
24 Micronutrients Phase 2, Phase 3
25 Trace Elements Phase 2, Phase 3
26 Protective Agents Phase 2, Phase 3
27 Vitamin B Complex Phase 2, Phase 3
28 Vitamins Phase 2, Phase 3
29 Folate Phase 2, Phase 3
30 Immunosuppressive Agents Phase 2, Phase 3
31 Antidotes Phase 2, Phase 3
32 Immunologic Factors Phase 2, Phase 3
33 Angiogenesis Inhibitors Phase 2, Phase 3
34 Antineoplastic Agents, Immunological Phase 2, Phase 3
35
Irinotecan Approved, Investigational Phase 2 100286-90-6, 97682-44-5 60838
36
Thalidomide Approved, Investigational, Withdrawn Phase 2 50-35-1 5426
37
Bortezomib Approved, Investigational Phase 2 179324-69-7 93860 387447
38
Cisplatin Approved Phase 2 15663-27-1 84093 441203 2767
39
Streptozocin Approved, Investigational Phase 2 18883-66-4 29327
40
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
41
Pantoprazole Approved Phase 2 102625-70-7 4679
42
Omeprazole Approved, Investigational, Vet_approved Phase 2 73590-58-6 4594
43
Gefitinib Approved, Investigational Phase 2 184475-35-2 123631
44
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
45
Clotrimazole Approved, Vet_approved Phase 2 23593-75-1 2812
46
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
47
Capecitabine Approved, Investigational Phase 2 154361-50-9 60953
48
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
49
Romidepsin Approved, Investigational Phase 2 128517-07-7 5352062
50
Everolimus Approved Phase 2 159351-69-6 6442177 70789204

Interventional clinical trials:

(show all 46)
# Name Status NCT ID Phase Drugs
1 Long-Term Study of the Efficacy and Safety of Lansoprazole in the Treatment of Zollinger-Ellison and Other Acid Hypersecretors Completed NCT00204373 Phase 4 Lansoprazole (Prevacid)
2 A Randomized, Double-blind, Placebo-controlled Multi-center Study to Evaluate the Safety and Efficacy of Fentanyl Sublingual Spray (Fentanyl SL Spray) for the Treatment of Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
3 A Phase III, Multicenter, Open-Label Study To Evaluate the Control of Gastric Acid Secretion With Esomeprazole In Patients With Gastric Acid Hypersecretory States Including Idiopathic Hypersecretion and Zollinger-Ellison Syndrome For 12 Months Completed NCT00079833 Phase 3 Esomeprazole magnesium (Nexium)
4 An Open-label, Parallel-group, Multi-centre, Phase I/III Study to Assess the Safety, Pharmacokinetics, Pharmacodynamics and Efficacy of Repeated Once-daily Oral Administration of D961H 10 mg and D961H 20 mg in Japanese Paediatric Patients 1 to 14 Years Old With Gastrointestinal Acid Related Diseases Completed NCT02153398 Phase 3 D961H sachet 10 mg;D961H capsule 10mg;D961H capsule 20 mg
5 A Pilot Study of FOLFOX in Combination With Bevacizumab in Patients With Advanced Neuroendocrine Tumors Terminated NCT00227617 Phase 2, Phase 3 5-fluorouracil;leucovorin;oxaliplatin
6 Study of First-Line Therapy Comprising Leucovorin Calcium, Fluorouracil, and Irinotecan (FOLFIRI) in Patients With Progressive Locally Advanced or Metastatic Duodenal-Pancreatic Endocrine Tumors Completed NCT00416767 Phase 2 fluorouracil;irinotecan hydrochloride;leucovorin calcium
7 Phase II Trial Of Thalidomide In Patients With Low Grade Neuroendocrine Tumors (Carcinoid and Islet Cell Cancers) Completed NCT00027638 Phase 2 thalidomide
8 Phase II Study of PS-341 in Metastatic Neuroendocrine Tumors Completed NCT00017199 Phase 2 bortezomib
9 A Phase II Clinical and Biologic Study of AMG 706 and Octreotide in Patients With Low-Grade Neuroendocrine Tumors Completed NCT00427349 Phase 2 AMG 706;octreotide
10 A Randomised Phase II Study Comparing Capecitabine Plus Streptozocin With or Without Cisplatin Chemotherapy as Treatment for Unresectable or Metastatic Neuroendocrine Tumors Completed NCT00602082 Phase 2 capecitabine;cisplatin;streptozocin
11 Evaluation of Treatment With Interferon, Octreotide, or Their Combination in Patients With Zollinger-Ellison Syndrome and Progressive Metastatic Non-B Islet Cell Neoplasm Completed NCT00001228 Phase 2 Interferon
12 Evaluation of Chemotherapy With Streptozotocin Combined With 5-Fluorouracil and Adriamycin in Patients With Zollinger-Ellison Syndrome and Metastatic Non-Beta-Islet Cell Neoplasm Completed NCT00001165 Phase 2 combined chemotherapy with streptozotocin, 5-fluorouracil, and doxorubicin
13 The Use of Oral Omeprazole and Intravenous Pantoprazole in Patients With Hypersecretion of Gastric Acid Completed NCT00001191 Phase 2 Pantoprazole;Omeprazole
14 An Open-label Volunteer Study of the Effect of G17DT on the Elevation of Plasma Gastrin Levels After Therapy With a Proton Pump Inhibitor (Omeperazole) in Patients With Treated Stage II/III Colorectal Cancer Completed NCT02518373 Phase 2 Omeprazole
15 A Phase II Trial of ZD1839 (Iressa®) in Metastatic Neuroendocrine Tumors Completed NCT00075439 Phase 2 gefitinib
16 Phase II Study of Single Agent Regorafenib in Patients With Advanced/Metastatic Neuroendocrine Tumors Completed NCT02259725 Phase 2 regorafenib
17 A Phase II Trial of Bay 43-9006 in Progressive Metastatic Neuroendocrine Tumors Completed NCT00131911 Phase 2 sorafenib tosylate
18 A Phase 2 Study of GW786034 (Pazopanib) in Advanced Low-Grade or Intermediate-Grade Neuroendocrine Carcinoma Completed NCT00454363 Phase 2 Pazopanib Hydrochloride
19 A Phase 2 Trial of Temsirolimus and Bevacizumab in Patients With Endometrial, Ovarian, Hepatocellular Carcinoma, Carcinoid or Islet Cell Cancer Completed NCT01010126 Phase 2 Temsirolimus
20 A Randomized Phase II Study of Temozolomide or Temozolomide and Capecitabine in Patients With Advanced Pancreatic Neuroendocrine Tumors Active, not recruiting NCT01824875 Phase 2 temozolomide;capecitabine
21 A Phase 2 Study of Capecitabine, Temozolomide, and Bevacizumab for Metastatic or Unresectable Pancreatic Neuroendocrine Tumors Active, not recruiting NCT01525082 Phase 2 Capecitabine;Temozolomide
22 Randomized Phase II Study of Everolimus Alone Versus Everolimus Plus Bevacizumab in Patients With Locally Advanced or Metastatic Pancreatic Neuroendocrine Tumors Active, not recruiting NCT01229943 Phase 2 Everolimus;Octreotide Acetate
23 Clinical Trial of Sir-Spheres® in Patients With Symptomatic or Progressive Hepatic Metastases From Neuroendocrine Tumors Terminated NCT00466856 Phase 2 octreotide acetate
24 A Pilot Trial of YF476, a Gastrin Antagonist, in Patients With Type II Gastric Carcinoids Associated With Zollinger-Ellison Syndrome Terminated NCT02454075 Phase 2 YF476
25 Phase II Study of Depsipeptide in Metastatic Neuroendocrine Tumors Terminated NCT00084461 Phase 2 romidepsin
26 A Randomized, Double-Blinded, Placebo-Controlled Phase II Study of Adjuvant Everolimus Following the Resection of Metastatic Pancreatic Neuroendocrine Tumors to the Liver Terminated NCT02031536 Phase 2 everolimus
27 Phase II Evaluation of Real-Time, Pharmacokinetically Guided Everolimus in Patients With Hormone Receptor Positive Breast Cancer, Pancreatic Neuroendocrine Tumors (PNET), and Renal Cell Carcinoma Terminated NCT02273752 Phase 2 Everolimus
28 Phase II Study of Vatalanib and Octreotide in Patients With Progressive Low-Grade Neuroendocrine Tumors Withdrawn NCT00227773 Phase 2 octreotide acetate;vatalanib
29 Phase II Study of Dovitinib (TKI-258) in Progressive, Well-Differentiated Pancreatic Neuroendocrine Tumors With and Without Prior VEGF-Inhibitor Therapy Withdrawn NCT02108782 Phase 2 dovitinib lactate
30 A Phase I, Pharmacokinetic, and Biologic Correlative Study of R115777 (NSC 702818) and Herceptin in Patients With Advanced Cancer Completed NCT00005842 Phase 1 tipifarnib
31 A Phase I Study of Intraperitoneal Oxaliplatin Alone and in Combination With Intraperitoneal Floxuridine and Leucovorin in Patients With Advanced Metastatic Cancer Confined to the Peritoneal Cavity Completed NCT00005860 Phase 1 floxuridine;leucovorin calcium;oxaliplatin
32 A Phase I, Open-Label, Maximum Tolerated Single-Cycle and Four-Cycle Dose-Finding Study to Evaluation the Safety and Tolerability of 90Y-SMT 487 Administered by Intravenous Infusion to Subjects With Refractory Somatostatin-Receptor Positive Tumors Completed NCT00006368 Phase 1
33 A Phase I, Open Label, Maximum Tolerated Dose-Finding Study to Evaluate the Safety and Tolerability of 90Y-DOTA-tyr3-Octreotide Administered by Intravenous Infusion to Children With Refractory Somatostatin-Receptor Positive Tumors Completed NCT00049023 Phase 1
34 A Phase I Trial of the mTOR Inhibitor RAD001 in Combination With VEGF Receptor Tyrosine Kinase Inhibitor PTK787/ZK 222584 in Patients With Advanced Solid Tumors Completed NCT00655655 Phase 1 everolimus;vatalanib
35 A Phase I Trial of Herceptin and Interleukin-12 Completed NCT00004074 Phase 1
36 Phase I Study of Anti-IGF-1R Monoclonal Antibody, IMC-A12, and mTOR Inhibitor, Everolimus, in Advanced Low to Intermediate Grade Neuroendocrine Carcinoma Completed NCT01204476 Phase 1 Everolimus;Octreotide Acetate
37 Phase I Feasibility Trial To Study The Safety Of Sealing Resected Pancreatic Surfaces After Partial Distal Pancreatectomy Using Laser Tissue Welding Recruiting NCT03147768 Phase 1
38 A Phase I Study of [111In-DTPA-D-Phe]-Octreotide in Patients With Refractory Malignancies Expressing Somatostatin Receptors Terminated NCT00002947 Phase 1
39 A Phase 1 Study of Veliparib (ABT-888) in Combination With Capecitabine and Temozolomide in Advanced Well-Differentiated Neuroendocrine Tumors Withdrawn NCT02831179 Phase 1 Capecitabine;Temozolomide;Veliparib
40 Clinical and Psychometric Validation of a Disease-Specific Questionnaire Module in Assessing the Quality of Life of Patients With G.I.-Related Neuroendocrine Tumours Unknown status NCT00454376
41 Evaluation of the Gastrinoma in Patients With Zollinger-Ellison Syndrome Completed NCT00001254
42 Medical Therapy of Zollinger-Ellison Syndrome Completed NCT00001241
43 Nexium Capsules Clinical Experience Investigation Completed NCT01434485
44 Diagnostic Evaluation of Patients With Suspected Abnormalities of Gastric Secretion Completed NCT00001240
45 Evaluation of the Safety and Sensitivity of 68Ga-DOTATOC PET/CT for Imaging NET Patients Recruiting NCT03583528
46 Treatment of Patients With Hepatic Neuroendocrine Metastases Using Drug-Eluting Bead Embolization Terminated NCT00730483 PVA microporous hydrospheres/doxorubicin hydrochloride

Search NIH Clinical Center for Zollinger-Ellison Syndrome

Inferred drug relations via UMLS 70 / NDF-RT 51 :


Cimetidine
Cimetidine Hydrochloride
Famotidine
lansoprazole
Nizatidine
Omeprazole
Omeprazole 2 MG/ML Oral Suspension
Omeprazole magnesium
pantoprazole
Pantoprazole sodium
rabeprazole

Cochrane evidence based reviews: zollinger-ellison syndrome

Genetic Tests for Zollinger-Ellison Syndrome

Anatomical Context for Zollinger-Ellison Syndrome

MalaCards organs/tissues related to Zollinger-Ellison Syndrome:

40
Pancreas, Pituitary, Lymph Node, Small Intestine, Liver, Pancreatic Islet, Thyroid

Publications for Zollinger-Ellison Syndrome

Articles related to Zollinger-Ellison Syndrome:

(show top 50) (show all 2935)
# Title Authors PMID Year
1
Diagnostic efficacy of the secretin stimulation test for the Zollinger-Ellison syndrome: an intra-individual comparison using different dosages in patients and controls. 61 54
20299818 2010
2
[Zollinger-Ellison syndrome: a case report.]. 61 54
20423792 2010
3
False-positive secretin stimulation test for gastrinoma associated with the use of proton pump inhibitor therapy. 61 54
19245850 2009
4
Regression of a large malignant gastrinoma on treatment with Sandostatin LAR: a case report. 61 54
18376130 2008
5
Secretin-receptor and secretin-receptor-variant expression in gastrinomas: correlation with clinical and tumoral features and secretin and calcium provocative test results. 54 61
17711922 2007
6
Chromogranin A: is it a useful marker of neuroendocrine tumors? 54 61
17513802 2007
7
Gastrinoma--morphological aspects. 61 54
17985091 2007
8
Diagnosis and differential diagnosis of hypergastrinemia. 54 61
17985088 2007
9
Increased islet beta cell replication adjacent to intrapancreatic gastrinomas in humans. 54 61
17016695 2006
10
Review article: Pathogenesis and management of gastric carcinoid tumours. 54 61
17059512 2006
11
Sporadic versus hereditary gastrinomas of the duodenum and pancreas: distinct clinico-pathological and epidemiological features. 61 54
17006979 2006
12
[Pseudo Zollinger-Ellison syndrome in a patient with duodenal stenosis caused by tuberculosis]. 54 61
16505764 2005
13
Double doses of secretin contribute to diagnosis of Zollinger-Ellison syndrome in secretin and selective arterial secretion injection tests--a case report. 61 54
16240211 2005
14
Precursor lesions in patients with multiple endocrine neoplasia type 1-associated duodenal gastrinomas. 61 54
15887103 2005
15
Intravenous calcium injection test is a novel complementary procedure in differential diagnosis for gastrinoma. 61 54
12205549 2002
16
The serological gastric biopsy: a non-endoscopical diagnostic approach in management of the dyspeptic patient: significance for primary care based on a survey of the literature. 54 61
12408500 2002
17
Prospective study of the natural history of gastrinoma in patients with MEN1: definition of an aggressive and a nonaggressive form. 54 61
11701693 2001
18
A randomized controlled crossover study comparing synthetic porcine and human secretins with biologically derived porcine secretin to diagnose Zollinger-Ellison Syndrome. 54 61
11328261 2001
19
Helicobacter pylori effects on gastritis, gastrin and enterochromaffin-like cells in Zollinger-Ellison syndrome and non-Zollinger-Ellison syndrome acid hypersecretors treated long-term with lansoprazole. 61 54
11136282 2001
20
Curative resection of microgastrinomas based on the intraoperative secretin test. 61 54
11038217 2000
21
Bleeding from alimentary tract in pseudo Zollinger-Ellison syndrome. 54 61
11208377 2000
22
Ovarian mucinous cystadenocarcinoma as a cause of Zollinger-Ellison syndrome: report of a case and review of the literature. 61 54
10811352 2000
23
Histomorphological characteristics of gastric mucosa in patients with Zollinger-Ellison syndrome or autoimmune gastric atrophy: role of gastrin and atrophying gastritis. 54 61
10738314 2000
24
Fundic gland polyps: a still elusive entity on the eve of the year 2000. 61 54
10833897 2000
25
The effect of Zollinger-Ellison syndrome and neuropeptide-secreting tumors on the stomach. 61 54
10980995 1999
26
Prospective study of the value of serum chromogranin A or serum gastrin levels in the assessment of the presence, extent, or growth of gastrinomas. 54 61
10193936 1999
27
Pancreatic endocrine tumors: recent advances. 61 54
10436815 1999
28
[The coexistence of 2 different neuroendocrine tumors of the upper gastrointestinal tract and pancreas]. 54 61
9861888 1998
29
Relationship between fundic endocrine cells and gastric acid secretion in hypersecretory duodenal ulcer diseases. 61 54
9726392 1998
30
Hypergastrinemia and enterochromaffin-like cell hyperplasia. 61 54
10461360 1998
31
[Gastric neuroendocrine tumor]. 61 54
10068736 1998
32
Management of patients and subjects at risk for multiple endocrine neoplasia type 1: MEN 1. GENEM 1. Groupe d'Etude des Néoplasies Endocriniennes Multiples de type 1. 54 61
9167954 1997
33
Use of calcium provocative test in the diagnosis of gastroenteropancreatic endocrine tumors. 61 54
9063035 1996
34
Adenocarcinoid of the liver arising within an area of hamartoma with predominant bile duct component. 54 61
8877646 1996
35
Prolonged hypergastrinemia does not increase the frequency of colonic neoplasia in patients with Zollinger-Ellison syndrome. 61 54
8617144 1996
36
Serum gastrin and secretin test in Zollinger-Ellison syndrome. 61 54
8551921 1996
37
Metastatic gastrinomas: localization with selective arterial injection of secretin. 54 61
8539410 1996
38
Endocrine tumors of the small and large intestine. 54 61
7479353 1995
39
Accuracy and precision of serum gastrin measurements in commercial laboratories. 61 54
8520176 1995
40
Treatment of peptic ulcers from now to the millennium. 54 61
7949462 1994
41
Laboratory medicine in ulcer disease. 61 54
7923753 1994
42
Gastrinomas demonstrate amplification of the HER-2/neu proto-oncogene. 61 54
7911296 1994
43
[Somatostatin receptor scintigraphy in neuroendocrine tumors exemplified by a patient with hepatic metastases of gastrinoma]. 54 61
7513113 1994
44
The pathology of the gastrointestinal endocrine system. 61 54
8125073 1993
45
Parenteral control of gastric acid hypersecretion in patients with Zollinger-Ellison syndrome. 61 54
8104773 1993
46
Management of patients with the Zollinger-Ellison syndrome. 54 61
7901311 1993
47
Use of selective arterial secretin injection test to guide surgery in patients with Zollinger-Ellison syndrome. 61 54
8362526 1993
48
Ovarian cancers express and process progastrin. 54 61
8467501 1993
49
[Zollinger-Ellison syndrome with basal gastrin and non-diagnostic stimulation tests (secretin and calcium)]. 61 54
8429704 1993
50
Chromogranin A and pancreastatin-like immunoreactivity in serum of gastrinoma patients. 61 54
8323758 1993

Variations for Zollinger-Ellison Syndrome

Expression for Zollinger-Ellison Syndrome

Search GEO for disease gene expression data for Zollinger-Ellison Syndrome.

Pathways for Zollinger-Ellison Syndrome

Pathways related to Zollinger-Ellison Syndrome according to KEGG:

36
# Name Kegg Source Accession
1 Gastric acid secretion hsa04971

GO Terms for Zollinger-Ellison Syndrome

Cellular components related to Zollinger-Ellison Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.56 SST SCT GRP GHRH GAST EGF
2 extracellular region GO:0005576 9.28 SST SCT NTS MLN GRP GHRH

Biological processes related to Zollinger-Ellison Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 response to food GO:0032094 9.4 GHRH GAST
2 cellular sodium ion homeostasis GO:0006883 9.37 ATP4A ATP12A
3 sodium ion export across plasma membrane GO:0036376 9.32 ATP4A ATP12A
4 cellular potassium ion homeostasis GO:0030007 9.26 ATP4A ATP12A
5 G protein-coupled receptor signaling pathway GO:0007186 9.23 SST SCT NTS MLN HRH2 GRP
6 establishment or maintenance of transmembrane electrochemical gradient GO:0010248 9.16 ATP4A ATP12A
7 positive regulation of phospholipase C-activating G protein-coupled receptor signaling pathway GO:1900738 8.96 GRP CHGA

Molecular functions related to Zollinger-Ellison Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 sodium:potassium-exchanging ATPase activity GO:0005391 9.32 ATP4A ATP12A
2 potassium-transporting ATPase activity GO:0008556 9.26 ATP4A ATP12A
3 potassium:proton exchanging ATPase activity GO:0008900 9.16 ATP4A ATP12A
4 neuropeptide hormone activity GO:0005184 9.13 NTS GRP GHRH
5 hormone activity GO:0005179 9.02 SST SCT MLN GHRH GAST

Sources for Zollinger-Ellison Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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